The Activity of Menkes Disease Protein ATP7A Is Essential for Redox Balance in Mitochondria

Activation of liver X receptor/retinoid X receptor pathway ameliorates liver disease inAtp7B−/−(Wilson disease) mice

(2016) James P. Hamilton et al.   HEPATOLOGY

Elevated copper impairs hepatic nuclear receptor function in Wilson’s disease

(2015) Clavia Ruth Wooton-Kee et al.   JOURNAL OF CLINICAL INVESTIGATION

X-linked spinal muscular atrophy in mice caused by autonomous loss of ATP7A in the motor neuron

(2015) Victoria L Hodgkinson et al.   JOURNAL OF PATHOLOGY

PET Imaging Analysis with 64Cu in Disulfiram Treatment for Aberrant Copper Biodistribution in Menkes Disease Mouse Model

(2014) S. Nomura et al.   JOURNAL OF NUCLEAR MEDICINE

Cellular glutathione plays a key role in copper uptake mediated by human copper transporter 1

(2013) Edward B. Maryon et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Maternal choline modifies fetal liver copper, gene expression, DNA methylation, and neonatal growth in the tx-j mouse model of Wilson disease

(2013) Valentina Medici et al.   Epigenetics

COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux

(2013) Scot C. Leary et al.   MOLECULAR BIOLOGY OF THE CELL

RNA-Guided Human Genome Engineering via Cas9

(2013) P. Mali et al.   SCIENCE

Targeted Chemical-Genetic Regulation of Protein Stability In Vivo

(2012) Susana Rodriguez et al.   CHEMISTRY & BIOLOGY

A structural model of the copper ATPase ATP7B to facilitate analysis of Wilson disease-causing mutations and studies of the transport mechanism

(2012) Maya Schushan et al.   Metallomics

A systems approach implicates nuclear receptor targeting in the Atp7b−/− mouse model of Wilson's disease

(2012) Phillip A. Wilmarth et al.   Metallomics

Measuring EGSH and H2O2 with roGFP2-based redox probes

(2011) Bruce Morgan et al.   FREE RADICAL BIOLOGY AND MEDICINE

Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease

(2011) Hans Zischka et al.   JOURNAL OF CLINICAL INVESTIGATION

ATP7A-related copper transport diseases—emerging concepts and future trends

(2011) Stephen G. Kaler   Nature Reviews Neurology

Hydrogen Peroxide Probes Directed to Different Cellular Compartments

(2011) Mikalai Malinouski et al.   PLoS One

Cellular copper levels determine the phenotype of the Arg875 variant of ATP7B/Wilson disease protein

(2011) A. Gupta et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An investigation of the effects of MitoQ on human peripheral mononuclear cells

(2010) Shiva Marthandan et al.   FREE RADICAL RESEARCH

Wilson Disease at a Single Cell Level

(2010) Martina Ralle et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Elevated Copper Remodels Hepatic RNA Processing Machinery in the Mouse Model of Wilson's Disease

(2010) Jason L. Burkhead et al.   JOURNAL OF MOLECULAR BIOLOGY

Effects of Copper Supplementation on the Structure and Content of Elements in Kidneys of Mosaic Mutant Mice

(2009) Małgorzata Lenartowicz et al.   BIOLOGICAL TRACE ELEMENT RESEARCH

Mammalian copper-transporting P-type ATPases, ATP7A and ATP7B: Emerging roles

(2009) Sharon La Fontaine et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Transcriptomic responses of European flounder (Platichthys flesus) to model toxicants

(2008) T WILLIAMS et al.   AQUATIC TOXICOLOGY

Real-time imaging of the intracellular glutathione redox potential

(2008) Marcus Gutscher et al.   NATURE METHODS

Investigating copper-regulated protein expression in Menkes fibroblasts using antibody microarrays

(2008) Tai Du et al.   PROTEOMICS

Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease

(2007) Eve A. Roberts et al.   MOLECULAR GENETICS AND METABOLISM